An 18-year-old man with Goodpasture's syndrome and ANCA-negative central nervous system vasculitis.
An 18-year-old man with Goodpasture's syndrome developed a recurrent seizure disorder refractory to anticonvulsant therapy spanning a full course of plasmapheresis and cytotoxic therapy. Status epilepticus developed during a taper of cytotoxic agents, with magnetic resonance imaging (MRI) findings consistent with multiple lacunar infarcts. Although the diagnosis of vasculitis was considered, anti-neutrophilic cytoplasmic antibody serology was consistently negative. A meningeal biopsy showed vasculitis, which was successfully treated with the reintroduction of high-dose steroids and cytotoxic agents in conjunction with anticonvulsants. Nearly 1 year later, with Goodpasture's disease in remission, he received a living-related renal transplant from his mother. He is currently leading a normal lifestyle. From an extensive review of the literature, this is the first case of anti-neutrophil cytoplasmic antibody-negative central nervous system (CNS) vasculitis in a patient with Goodpasture's syndrome.
Rydel
J J
JJ
Department of Nephrology, Christ Hospital Center, Oak Lawn, IL, USA. nephron1@aol.com
Rodby
R A
RA
D000293:Adolescent; D019867:Anti-Glomerular Basement Membrane Disease / Q000150:complications* / Q000276:immunology; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis*; D001706:Biopsy; D001921:Brain / Q000473:pathology; D002493:Central Nervous System Diseases / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D007668:Kidney / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008578:Meninges / Q000473:pathology; D012640:Seizures / Q000209:etiology; D014657:Vasculitis / Q000150:complications* / Q000175:diagnosis
D019268:Antibodies, Antineutrophil Cytoplasmic